Risk of SUDEP during infancy.

ABSTRACT

Risk of sudden unexpected death in epilepsy (SUDEP) in children is influenced by different factors such as etiology, seizure type and frequency, treatment, and environment. A greater severity of epilepsy, in terms of seizure frequency, seizures type, especially with nocturnal generalized tonic-clonic seizures (GTCS), and resistance to anti-seizure medication are predisposing factors to SUDEP. Potential mechanisms of SUDEP might involve respiratory, cardiovascular, and central autonomic dysfunctions, either combined or in isolation. Patients with epilepsy carrying mutations in cardiac channelopathy genes might be disposed to seizure-induced arrhythmias. Other than in channelopathies, SUDEP has been reported in further patients with genetic epilepsies due to mutations of genes such as DEPDC5, TBC1D24, FHF1, or 5q14.3 deletion. Age-related electro-clinical differences in GTCS may therefore be relevant in explaining differences in SUDEP between adults and children. Typical GTCS represent a rare seizure type in infants and toddlers, they are characterized by a shorter tonic phase and, in direct proportion, by shorter postictal generalized EEG suppression (PGES). The presence of night-time supervision has been found to reduce SUDEP risk, likely reducing SUDEP incidence in children. Reconsideration of safety protocols in epilepsy monitoring units with the aim of reducing the risk of SUDEP, and the use of devices for seizure detection, might contribute to reduce the risk of death in patients affected by epilepsy. This article is part of the Special Issue "Severe Infantile Epilepsies".